Hepatopulmonary syndrome (HPS), which consists of intrapulmonary vascular dilatation and impaired oxygenation in the setting of liver dysfunction or portal hypertension (1, 2), has predominantly been reported in patients with cirrhosis. We evaluated the frequency of this syndrome in noncirrhotic portal hypertension (1, 3).

Twenty-five consecutive patients (16 men) with biopsy-proven noncirrhotic portal hypertension (24 with noncirrhotic portal fibrosis and 1 with portal-vein thrombosis) without cardiopulmonary disease were screened by air contrast–enhanced echocardiography. When results were positive, arterial blood gas analysis and pulmonary function tests were conducted. The HPS was diagnosed by positive contrast echocardiogram and a Pao2 less than 70 mm Hg or a standing alveolar–arterial gradient greater than 20 mm Hg (1, 4).